Review Article

Sarcoma Surveillance: A Review of

Current Evidence and Guidelines

Cara A. Cipriano, MD, MS
Eugene Jang, MD, MS
Wakenda Tyler, MD, MPH
Abstract
After initial treatment of sarcoma, disease progression may occur in
the form of local recurrence, pulmonary metastases, or
extrapulmonary metastases. As such, surveillance is an important
aspect of management, but no universally accepted practice
standards are found. In the absence of strong evidence, and to allow
for individualized care, existing guidelines contain flexibility in terms of
both the frequency and modality of surveillance. In general, they agree
that follow-up should be more intense in the early years after
treatment, especially for high-grade sarcomas, and continue for at
least 10 years. For local recurrence, data suggest that physical
examination is usually sufficient for monitoring; in addition, some
guidelines endorse imaging routinely, whereas others only as
clinically indicated. For pulmonary metastasis, either radiograph or CT
is recommended, with the latter having theoretical advantages but no
proven survival benefit to date. Extrapulmonary metastases are rare in
most sarcoma types, so the literature only supports extrapulmonary
surveillance for certain diagnoses. This topic is complicated by the
diversity of sarcomas, the limited evidence, and the indefinite, often
conflicting recommendations; therefore, it is critical for providers to
understand the existing research and guidelines to determine optimal
surveillance strategies for their patients.

From the Washington University in St

Louis (Dr. Cipriano), St Louis, MO,
and Columbia University Medical
Center (Dr. Jang and Dr. Tyler), New
York, NY.
None of the following authors or any
immediate family member has
received anything of value from or has
stock or stock options held in a
commercial company or institution
related directly or indirectly to the
subject of this article: Dr. Cipriano,
Dr. Jang and Dr. Tyler.
J Am Acad Orthop Surg 2020;28:
145-156
DOI: 10.5435/JAAOS-D-19-00002
Copyright 2019 by the American
Academy of Orthopaedic Surgeons.
S arcomas are a diverse group of
over 50 malignancies of mesen-
chymal origin. They arise as primary
tumors of the musculoskeletal system
and affect patients of all ages. The
incidence of sarcoma in the United
States was estimated at 15,000 cases
in 2014, and although it has been
increasingly diagnosed in recent dec-
ades, it remains a relatively rare dis-
ease. Five-year mortality for sarcoma
in general is approximately 35% but
varies greatly based on the disease
type; most low-grade tumors
have minimal risk, whereas some
high-grade tumors are almost uni-
versally fatal.
Primary, nonmetastatic sarcomas
are usually managed with some
combination of chemotherapy, radi-
ation therapy, and surgical resection.
Although these initial treatments are
curative in many cases, disease pro-
gression can occur in the form of local
recurrence (LR) and/or distant
metastases. LR is problematic
because it can cause symptoms and
require additional, often more com-
plex surgical procedures. Metastatic
disease, which can develop in the
presence or absence of LR, is the
major cause of mortality associated
with sarcoma. Metastases most
commonly affect the lungs but can
also occur in other viscera, soft tis-
sues, or bones. Detecting disease
progression early has the potential to
reduce morbidity and mortality in

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Sarcoma Surveillance

Figure 1

Graphs showing the rates of local recurrence (LR) for high- and low-grade soft-tissue sarcoma (STS). A, For high-grade
STS, the incidence of LR is highest in the first 2 years after treatment. B, For low-grade soft-tissue sarcoma, the rate is more
constant, with late recurrences occurring more frequently than in high-grade tumors.5 Although higher grades of STS have
shown a clear association with higher rates of LR, the evidence to date for primary bone sarcomas has not revealed a similar
link between grade and the incidence of LR.6

certain cases, making surveillance an
important aspect of management.
Despite this fact, the optimal
imaging modality and frequency for
surveillance remains uncertain. The
rarity of sarcomas, combined with
the broad range of disease types and
patients affected, makes this a chal-
lenging cohort to study and treat.
Higher frequency surveillance with
more advanced imaging techniques
may detect disease recurrence
sooner and, thus, facilitate treat-
ment. Conversely, disadvantages of
more intense surveillance include
increased time for physicians and
patients, radiation exposure, and
risk of false-positive results requir-
ing costly and unnecessary addi-
tional workup. Resource utilization
is also a notable consideration, as
the expense associated with sur-
veillance can vary dramatically
with frequency and modality; for
example, a review in 2004
describes a total of 54 different
surveillance strategies for soft-
tissue sarcomas (STS) with charges
ranging from $485 to $21,235, a
42.8-fold cost differential.1
Owing to the complexity of this
topic, no universal practice standards
are found with respect to surveillance
for LR, pulmonary metastases (PMs),
or extrapulmonary metastases (EMs).
The existing guidelines contain flexi-
bility in both the recommended fre-
quency and modality of surveillance;
physicians must therefore understand
the options to determine how to
follow-up their patients. The pur-
pose of this article is to review the
available evidence and consensus-
based guidelines on sarcoma sur-
veillance to help inform these
decisions.
Review of Evidence
Local Recurrence
Surveillance: Considerations
LR after treatment of sarcomas in the
extremities is associated with
increased morbidity and mortality.
Re-excision of LR can be a
relatively minor procedure if the
recurrence is detected early, but
larger recurrences can require a
major operation involving complex
reconstruction or amputation. The
STS literature also suggests that the
occurrence of LR independently pre-
dicts higher rates of subsequent
metastasis and reduced overall sur-
vival.2 However, if LR can be de-
tected and treated before metastasis,
patients’ overall survival is markedly
better than if metastasis has already
occurred.3 Thus, surveillance for LR
can theoretically improve survival
and reduce morbidity of treatment.
A number of risk factors are
associated with the incidence of LR
after sarcoma and, therefore, justify
more intense local surveillance. Time
since initial treatment is a strong
predictive factor for both bone sar-
coma and STS; most LRs take place
within the first 5 years, and frequency
continues to decline with time.4,5
Histologic grade of the tumor also
affects the LR rate in extremity soft-
tissue and bone sarcomas (Figures 1
and 2). A retrospective study of 105
patients with STS by Sugiura et al3
identified tumors deep to the fascia
or located in the upper
extremity/trunk as higher risk of LR.
In addition, several studies have

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 Cara A. Cipriano, MD, MS, et al

found that close or positive margins Figure 2

are strongly associated with the risk
of LR as well as higher mortality in
both bone sarcoma and STS,
although the precise definition of an
appropriate margin remains
debated.2,3,8,9 When positive mar-
gins result from unplanned re-
sections, timely and appropriate
treatment with an adequate wide
resection results in a return to the
baseline rate of LR; however, the
associated surgical morbidity is often
higher.10,11 Local contamination
as a result of tumor seeding during
biopsy can also potentially contrib-
ute to LR. Barrientos-Ruiz et al12
histologically examined biopsy tract
resections and found a higher inci-
dence of contamination (cell seeding)
in cases of open compared with
percutaneous biopsies; moreover,
they observed a higher rate of LR in Kaplan-Meier plot demonstrating the rates of local recurrence (LR) for high-,
the cases with evidence of contami- intermediate-, and low-grade bone sarcomas. LR appeared to occur more
nation. Conversely, Binitie et al13 frequently in higher grade sarcomas during the first 3 years after treatment, but
with the number available for study no notable differences were found between
found no difference in disease-free rates of LR among tumor grades. LR after 5 years was rare, and no instances of
survival when the tract of a percu- LR of low-grade sarcomas beyond 4 years were found.7
taneous biopsy was not excised, as
long as patients subsequently
received radiation. Taken together, cially for STS, although its benefit Pulmonary Metastasis
these findings suggest that percuta- over physical examination has not Surveillance: Considerations
neous biopsy combined with radia- been established. Several studies
The lungs are the most common site
tion does not produce clinically have suggested that the majority of
of sarcoma metastases, and as such,
significant local contamination, but LR are identified by patients, and
that MRI should be reserved for PMs have notable implications on
open biopsy can potentially increase
tumors not easily evaluated by mortality.18 Studies have found that
the risk of LR.
physical examination.14-16 Bone in STS, the presence of .5 PMs at
scan is not applicable for STS and time of diagnosis markedly reduces
Local Recurrence nonspecific for bone sarcomas, median survival (22 versus
19
55 months), and the number and
Surveillance: Modalities especially after skeletal reconstruc-
Several approaches to monitoring for tion. Finally, positron-emission distribution of subcentimeter lung
local recurrence have been proposed. tomography (PET) in conjunction nodules predicts survival in young
Physical examination remains an with CT is often used for staging sarcoma patients.20 A growing body
essential component of surveillance, purposes and has also been assessed of evidence supports the role of in-
and patient self-examination has as a surveillance tool. The evidence terventions for limited PMs as a
been shown to be highly effective.14 to date does not demonstrate a means of improving survival in both
Radiographs are a relatively low- notable difference between receiver bone sarcoma and STS.21,22 The
cost and low-risk imaging modality operating characteristic curves of potential to improve survival
that represents an important means PET/CT versus MRI for detecting through early detection of PM may
of identifying LR of bone sarcomas. LR of STS.17 These data, combined justify the practice of intense chest
However, they are less effective in with its increased cost, do not surveillance for patients who are
detecting soft-tissue LR.4 MRI is support the use of PET imaging for likely to benefit from treatment.
therefore also heavily used, espe- routine surveillance at this time. However, the prognosis of

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Sarcoma Surveillance

Figure 3

Graphs showing the rates of pulmonary metastasis (PM) for high- and low-grade soft-tissue sarcoma (STS). A, For high-
grade STS, the incidence of pulmonary metastasis was markedly higher in the initial 2 years after treatment. B, For low-
grade STS, the incidence was more constant over time, with late PM occurring at higher rates than low-grade tumors.5

metastatic sarcoma remains poor,

Figure 4
and many patients are not eligible for
curative treatments, and so, in these
situations, the benefits of surveil-
lance are less clear.
Generally, established risk factors
for sarcoma PM include high histo-
logic grade, location deep to the fas-
cia, and larger tumor size. Additional
risk factors have been described for
patients with STS (close/positive
margins, LR after appropriate resec-
tion, increased patient age, male sex,
and ,90% necrosis after preopera-
tive treatment)2,3 as well as osteo-
sarcoma (tumor location, histologic
response to chemotherapy, patient
age, and laboratory values such as
alkaline phosphatase).23,24 As with
LR, the time from surgery influences
the risk of PM. For both soft-tissue
and bone sarcomas, high-grade tu-
Kaplan-Meier plot demonstrating the rates of pulmonary metastasis (PM) for
high-, intermediate-, and low-grade bone sarcomas. Higher metastatic rates
mors are most likely to metastasize
were observed in higher grade sarcomas. Although the frequency of events within 2 years of follow-up,
generally decreased over time for all groups, most PMs for high grade occurred whereas low-grade tumors metas-
within the first 2 years, whereas the rate was more stable for low- and tasize at a lower and more constant
intermediate-grade sarcomas. New metastases were rare after 4 years for low-
grade tumors and after 10 years for intermediate- or high-grade tumors. Of note, rate (Figures 3 and 4). Thus, to
osteosarcomas (OSA) that were histologically classified as intermediate grade maximize the yield of PM detection
were noted to follow the pattern of high-grade sarcomas and were therefore per examination, the frequency of
considered grade 3 in this analysis.7
pulmonary surveillance should be

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dictated by the tumor grade and
time since treatment.7
Pulmonary Metastasis
Surveillance: Modalities
The use of radiograph versus CT for
pulmonary surveillance is a highly
debated topic in orthopaedic oncol-
ogy. Chest CTs, when compared with
chest radiographs, offer the ability to
detect smaller lung metastases; how-
ever, the clinical relevance of this
sensitivity has been questioned. Some
studies have shown that the increased
sensitivity of chest CT translates
into a statistically significant increase
in overall survival for osteosarcoma
PM.25 By contrast, level I evidence
published by Puri et al demonstrated
no disease-free or overall survival
benefit from CT compared with
radiograph; in addition, in a follow-
up study, they found no advantage
to imaging every 6 versus
3 months.14,26
CT scans are also not without
controversy and risk. The increased
sensitivity of chest CT is also accom-
panied by a higher false-positive rate,
with a 33% rate of finding false-
positive nodules after two annual
chest CTs, versus just 15% when
using chest radiograph over the same
period.27 Furthermore, radiation
exposure from a CT scan is two or-
ders of magnitude higher than
radiograph and may pose a notable
cumulative risk of malignancy.28 In a
2016 Musculoskeletal Tumor Soci-
ety (MSTS) survey, 62.9% of re-
spondents indicated that patients
have expressed concerns regarding
radiation exposure from surveillance
within the past year.29 PET imaging
has been suggested as a means of
avoiding the radiation exposure of
CT for pulmonary surveillance, and
18-FDG-PET has been shown to have
high sensitivity and intermediate
specificity for lung nodules .1 cm.
However, PET is expensive and has
relatively low sensitivity for sub-
February 15, 2020, Vol 28, No 4
Copyright © the American Academy of Orthopaedic Surgeons. Unauthorized reproduction of this article is prohibited.
centimeter lung nodules,30 which
limits its utility for pulmonary sur-
veillance in its current technological
state.
Currently, the use of radiograph
versus CT varies according to train-
ing and the geographic region. For
example, European surgeons are
more likely to use chest radiographs
for surveillance, whereas surgeons in
the United States more frequently use
chest CT.29,31,32 Despite this practice
pattern of increased chest CT usage
in the United States, the results from
an MSTS survey indicated that one-
third of respondents felt that CT is
overutilized for high-grade tumors
and two-third felt CT is overutilized
for low-grade tumors.33 The use of
separate protocols for high- and low-
risk surveillance, with fewer routine
CTs for the latter, would potentially
be more cost effective, radiation
sparing, and noninferior in terms of
accuracy.32,34 The specific diagnosis
and patient characteristics should
also be taken into consideration, as
these factors impact whether early
detection of PM can improve out-
comes. For example, if effective
treatments are not available, or if the
patient is not eligible for existing
treatments due to age or comorbid-
ities, early detection of PM becomes
less clinically relevant, and a less
intense surveillance plan may be
justified.
Extrapulmonary Metastasis
Surveillance: Considerations
EMs from sarcoma are rare and are
associated with a worse prognosis
than PM alone (15 versus
38 months).19 Historically, the
presence of EM has been
considered a contraindication for
resection of PM, but recent research
suggests that resection of both PM
and EM results in survival
(37.8 months) that is similar to those
with only PM treated with lung
metastasectomy (35.5 months). Fur-
Cara A. Cipriano, MD, MS, et al
thermore, both of the aforementioned
groups had markedly improved sur-
vival when compared with those with
both PM and EM that only had lung
metastasectomy and left the EMs
untreated (13.5 months).33 There-
fore, timely diagnosis of EMs after
sarcoma treatment may have a tan-
gible effect on treatment choices and
patient survival.
Although the incidence and risk
factors for EM are not entirely
understood, higher grade STS is
associated with a higher incidence of
distant EM.35 Certain sarcoma his-
tologies also exhibit a greater pre-
dilection for EM; in particular,
myxoid-round cell liposarcoma is
known to metastasize to the retro-
peritoneum, abdominal wall, and
abdominal cavity.36,37 Solitary fibrous
tumors can also require long-term
surveillance because of benign-acting
masses reportedly transforming and
metastasizing decades after initial
presentation.38 Although hematologic
spread predominates in most sarco-
mas, regional lymphatic spread is
known to occur in synovial sarcoma,
angiosarcoma, rhabdomyosarcoma,
clear-cell chondrosarcoma, and epi-
thelioid sarcoma; therefore, ex-
trapulmonary monitoring may be
justified in these diagnoses. Finally,
distant EMs of osteosarcoma are
rare but have been described in the
bones, pleura, and heart. It has been
hypothesized that newer medical
treatments may be responsible for
the apparent increase in incidence of
EM of osteosarcoma in recent his-
tory.39 If this proves to be the case,
more routine extrapulmonary sur-
veillance may justified in certain
situations.
Pulmonary Metastasis
Surveillance: Modalities
Because relatively few indications are
noted for surveillance for EM aside
from the histologies listed previously,
no strong evidence exists to support the
149
Sarcoma Surveillance

Table 1
NCCN Guidelines for Surveillance of Soft-Tissue and Bone Sarcomas After Initial Treatment42,43
History and
Physical
Factor Type Examination Local Imaging Chest Imaging Other Modalities

Soft-tissue Stage I STS Q3-6 mo for 2–3 MRI with and Chest imaging US may be useful
sarcomas yr, then annually without contrast Q6-12 mo by for small,
and/or CT with CT (preferred) superficial
contrast as or radiograph tumors
clinically indicated,
unless the area is
easily followed by
physical
examination
Stage II-IV STS Q3-6 mo for 3 yr, Baseline Chest imaging US may be useful
then Q6 months postoperative MRI Q3-6 mo for 3 yr, for small,
for 4-5 yr, then with and without then Q6 months superficial tumors.
annually (preferred) or CT for 4-5 yr, then PET/CT may be
with contrast, then annually used to determine
periodically response to
depending on risk neoadjuvant
chemotherapy
with .3-cm firm/
deep lesions
Bone Osteosarcoma Q3 months for 2 yr, MRI or CT with Chest imaging CBC/other
sarcomas Q4 months for 3 yr, contrast Q3 Q3 months for 2 yr, laboratory values
Q6 months for 4- months for Q4 months for 3 yr, as clinically
5 yr, then annually 2 yr, Q4 months Q6 months for 4- indicated. Whole-
for 3 yr, Q6 5 yr, then annually body PET/CT
months for 4-5 yr, scan and/or bone
then annually scans can be
considered (level
2B evidence)
Ewing’s sarcoma Physical Q2-3 mo for 2 yr, Chest radiograph Whole-body PET/
examination and then increasing or CT Q2-3 mo for CT or bone scan
laboratory values intervals until 5 yr, 2 yr, then can be considered
Q2-3 mo for 2 yr, then annually increasing
then increasing indefinitely (level intervals until 5 yr,
intervals until 5 yr, 2B evidence for then annually
then annually long-term indefinitely (level
indefinitely (level surveillance) 2B evidence for
2B evidence for long-term
long-term surveillance)
surveillance)
Chordoma As clinically Radiographs, MRI, Chest imaging CT abdomen/
indicated or CT of the local Q6 months, may pelvis with
site as clinically include chest CT contrast annually
indicated annually for 5 yr,
then annually
thereafter
Low-grade/ As clinically Radiographs and/ Chest imaging as
intracompartmental indicated or cross-sectional clinically indicated
chondrosarcoma imaging with Q6-12 mo for 2 yr,
contrast as then yearly as
clinically appropriate
indicated for 2 yr,
then yearly as
appropriate
(continued )
CBC = complete blood count, NCCN = National Comprehensive Cancer Network, PET = positron-emission tomography, STS = soft-tissue sarcoma,
US = ultrasound

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Table 1 (continued )
NCCN Guidelines for Surveillance of Soft-Tissue and Bone Sarcomas After Initial Treatment42,43
Factor Type
Bone High-grade (II or III)/ As clinically
sarcomas clear-cell/
(continued) extracompartmental
chondrosarcoma
Giant cell tumor of
bone
CBC = complete blood count, NCCN = National Comprehensive Cancer Network, PET = positron-emission tomography, STS = soft-tissue sarcoma,
US = ultrasound
use of routine CT of the chest, abdo-
men, and pelvis in STS surveillance.40
Similarly, surveillance of ex-
trapulmonary sites is not generally
recommended for osteosarcoma,
although bone scans represent an
accurate methodology for detecting
the subsets of EM from osteosarcoma
that are calcified.41 There may be a
role for whole-body PET scan for
surveillance for EM in the future, but
as of yet limited evidence exists to
support this practice.
Review of Guidelines
Current guidelines for sarcoma sur-
veillance have been issued by organ-
izations including the National
Comprehensive Cancer Network
(NCCN), the European Society for
Medical Oncology (ESMO), the British
Sarcoma Group (BSG), and the MSTS
in conjunction with the American
Academy of Orthopaedic Surgeons
(MSTS/AAOS). In this section, we will
review these guidelines for both soft-
tissue and bone sarcomas and the levels
of evidence on which they are based.
National Comprehensive
Cancer Network
The 2018 NCCN guidelines for STS
management and surveillance42 are
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History and
Physical
Examination Local Imaging
Radiographs and/or Chest imaging Q3-6
indicated cross-sectional
imaging with
contrast as
clinically indicated then yearly for
As clinically MRI or CT with
indicated contrast as
clinically indicated then annually
based on level 2A evidence, and the
authors disclose that “very limited
data are available in the literature
on effective surveillance strategies”
for STS (Table 1). The NCCN
emphasizes risk-stratification for
surveillance, with increased attention
to patients with larger, high-grade
tumors in the early postoperative
years. For LR surveillance, they state
that physical examination may suf-
fice in some situations, and ultraso-
nography may be useful for
superficial tumors when performed
by an experienced technician. In all
other cases, the guidelines generally
recommend periodic MRI with and
without contrast or CT with contrast
based on the risk of disease pro-
gression. For chest imaging, the
NCCN prefers CT over radiograph
but acknowledges that no study has
demonstrated improved outcomes
with CT surveillance.
The NCCN guidelines for bone
sarcoma surveillance were pub-
lished in 2013 and revised in
2017.43,44 Similar to the soft-tissue
guidelines, they are based on rela-
tively low-level evidence supported
by expert consensus. Because of the
known variation in treatment
modalities and patterns of recur-
rence between specific diagnoses,
the guidelines were categorized
Cara A. Cipriano, MD, MS, et al
Chest Imaging Other Modalities
mo, may include
chest CT at least
Q6 months for 5 yr,
a minimum of 10 yr
Chest imaging
Q6 months for 2 yr,
thereafter
based on the four most common
bone tumors encountered (chon-
drosarcoma, osteosarcoma, chor-
doma, and Ewing’s sarcoma) and
giant cell tumor of bone, a locally
aggressive tumor that is considered
benign but has metastatic potential
(Table 1).43,44
European Society for Medical
Oncology
In the 2018 ESMO STS guide-
lines,45 the authors acknowledge
that few published data indicate
optimal guidelines for follow-up of
patients with STS and offer a range
of acceptable options as practical
guidelines (Table 2). Of note, they
state that use of sensitive advanced
imaging modalities (ie, MRI and
CT) may diagnose LR and PM
earlier; however, “it has not been
demonstrated that this is benefi-
cial, or cost effective, compared
with the clinical assessment of the
primary site and regular chest
X-rays.”45
For bone sarcomas, the most
recent ESMO guidelines (2014)46
again disclose that no clear con-
sensus exists among the expert
panel or in the literature to support
one definite practice before pro-
viding some general guidelines.
151
Sarcoma Surveillance

Table 2
EMSO Guidelines for Surveillance of Soft-Tissue and Bone Sarcomas After Initial Treatment45,46
History and Physical
Factor Type Examination Local Imaging Chest Imaging Other Modalities

Soft-tissue Low grade Q4-6 mo for 2 yr, Chest radiograph or

sarcomas less frequently CT at longer
until 5 yr, then intervals in the
annually first 3-5 yr, then
annually
Intermediate/ Q3-4 mo for 2–3 yr,
high grade Q6 months until 5 yr,
then annually
Bone Low grade Q6 month for 2 yr, MRI or CT both Chest radiograph or PET scan and bone
sarcomas then annually with contrast Q3 CT Q3 months for scans can be
mo for 2 yr, Q4 mo 2 yr, Q4 months for considered—lower
for 3 yr, Q6 mo for 4 3 yr, Q6 months for 4- level of evidence
and 5 yr, yearly 5 yr, then annually (2B)
thereafter
High grade Q2-3 mo for 2 yr, Nonspecific Q2-3 mo for 2 yr, Q2-4 Ewing’s sarcoma,
Q2-4 mo for 3-4 yr, mo for 3-4 yr, whole-body imaging
Q6 months for 5- Q6 months for 5- in the form of bone
10 yr, Q6-12 mo 10 yr, Q6-12 mo scan or PET scan
thereafter thereafter

EMSO = European Society for Medical Oncology, PET = positron-emission tomography

Table 3
BSG Guidelines for Surveillance of Soft-Tissue and Bone Sarcomas After Initial Treatment47,48
History and Physical Other
Factor Type Examination Local Imaging Chest Imaging Modalities

Soft-tissue Low grade Q4-6 mo for 3-5 yr, Based on clinical As indicated for patients who
sarcomas then annually until 10 yr indications would receive intervention if
metastatic disease was
detected early
Intermediate/ Q3-4 mo for 2-3 yr, Based on clinical Q3-4 mo for 2-3 yr, Q6 months
high grade Q6 months until 5 yr, indications until 5 yr, then annually until
then annually until 10 yr 10 yr
Bone Low grade Q4-6 mo for the first 2 yr, then Based on clinical
sarcomas annually indications
High grade Q2-4 mo for 3 yr, Q6 months Based on clinical
until 5 yr, then annually indications

BSG = British Sarcoma Group

Instead of specific recommendations
regarding intervals or modality, they
allow for variability depending on
local practice and other tumor- or
patient-related risk factors (Table 2).
Although they suggest decreasing
frequency of follow-up over time,
they do not specify an end point given
the potential for LR, metastases, and
functional deficits may occur .10
years after diagnosis.
British Sarcoma Group
In 2010 and 2016, the BSG published
recommendations for treatment and
follow-up of soft-tissue and bone sar-
comas based on expert opinion and
data collected from various sources,
including the NCCN and ESMO. In
the same manner as these other or-
ganizations, they point out the limi-
tations of current evidence on
sarcoma surveillance, and as such,
their guidelines are designed to be
general rather than prescriptive47,48
(Table 3). Of note, the group

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defines a standard follow-up visit as
including “(1) review of any new
symptoms reported by the patient,
(2) clinical examination to focus on
LR, with imaging follow-up where
indicated by clinical suspicion, (3)
routine chest radiograph to exclude
pulmonary metastases, and (4) mon-
itoring for late effects of treatment.”47
For STS, the BSG recommends
physical examination as the basis of
LR surveillance, with advanced imag-
ing based on clinical indications. They
suggest that chest imaging be reserved
for patients who would receive inter-
vention if metastatic disease was de-
tected early.47 In addition, of note,
they advocate for the use of chest
radiograph rather than CT for PM
surveillance. They recommend clinical
and chest follow-up more frequently
for high- than low-grade tumors,
although both groups should be fol-
lowed up for 10 years because of the
risk of late recurrences (Table 3).
BSG guidelines for bone sarco-
mas are similar to those for STS48
(Table 3). The authors indicated
that strong recommendations for
local imaging could not be made
and that this should be based on
clinical indications. In addition,
given recent evidence that patients
often identify their own recurrences,
the BSG surveillance plans include
patient education on self-examination
and appropriate measures to take if
symptoms suggest LR. Finally, owing
to the risk of late disease progression
and other long-term treatment com-
plications, the group likewise could
not recommend a definitive end point
for surveillance.48
Musculoskeletal Tumor
Society and American
Academy of Orthopaedic
Surgeons
In two recent survey studies of
MSTS members, .90% of re-
spondents expressed concerns
about the lack of evidence-based
February 15, 2020, Vol 28, No 4
Copyright © the American Academy of Orthopaedic Surgeons. Unauthorized reproduction of this article is prohibited.
guidelines for sarcoma surveil-
lance.29,49 In 2017, the MSTS
identified this as the organization’s
single highest research priority and
subsequently, in conjunction with
the AAOS, defined appropriate use
criteria to aid in clinical decision
making in this area.
The “surveillance of local recur-
rence and distant metastases after
surgical treatment of bone and soft-
tissue sarcomas” criteria were
developed by a panel of experts
representing orthopaedic oncology,
surgical oncology, medical oncology,
and musculoskeletal radiology. The
process was initially informed by a
systemic review of the literature on
the subject. A writing subset of the
group determined that the most
important criteria were risk of
metastatic disease, risk of LR, tumor
tissue of origin, extrapulmonary
metastatic risk, history of recurrence,
and time since treatment. They
developed 144 scenarios with vari-
ous combinations of these factors, as
well as 27 treatment options. The
remainder of the panel reviewed all
scenarios and voted on the most
appropriate imaging surveillance
modalities for each, after which the
entire group met to discuss areas of
discrepancy. The independent voting
process was then repeated, and
guidelines were formulated based on
the results, with the strength of the
recommendation correlating with the
degree of expert consensus. The final
document was approved by the
leadership of the MSTS and AAOS
and was incorporated into AAOS
OrthoGuidelines in 2018.
This decision-making aid can be
accessed through the AAOS Ortho-
Guidelines website under the Appro-
priate Use Criteria tab50 or on a
mobile device through the Ortho-
Guidelines application, available for
iOS and Android.51 When health-
care providers enter characteristics
of a specific patient scenario, the
program lists surveillance plans
Cara A. Cipriano, MD, MS, et al
(including frequency and modality)
that are “appropriate,” “may be
appropriate,” and “rarely appropri-
ate” in that context. Like the previ-
ously discussed guidelines, this tool
offers a range of options for sur-
veillance, allowing providers to
exercise clinical judgment in the
absence of strong evidence.
Summary
The various guidelines for sarcoma
surveillance that have been issued by
the international medical community
contain several similarities (Figure 5).
Common themes include more
intense surveillance in the early years
after treatment, especially for high-
grade sarcomas. The literature sug-
gests that physical examination is
sufficient to detect LR in most cases;
some guidelines recommend
advanced imaging routinely,
whereas others leave this to the dis-
cretion of the clinician. Evidence and
recommendations for the optimal
pulmonary surveillance modality
remain divided between radiograph
and CT, with the latter having the-
oretical advantages but no proven
survival benefit to date. Length of
follow-up is also not clearly defined,
although most guidelines recom-
mend surveillance for at least 10
years; after this time frame, disease
progression does occur, but at a
negligible rate.5 Additional consid-
erations that are not specifically ad-
dressed in the published guidelines
include cost to healthcare systems
(monetary and time expenditures)
and issues affecting patient quality of
life (logistical challenges and anxiety
related to testing and false-positive
results). Finally, as new therapies
become available, targeted surveil-
lance strategies may be indicated
based on the relative ability to
effectively treat different types of
metastatic sarcoma. For example,
more intense chest imaging may be
153
Sarcoma Surveillance

Figure 5

Summary of surveillance protocols recommended by the NCCN,42,43 ESMO,45,46 and BSG,47,48 with suggested frequencies
for obtaining history and physical examination (H&P), local imaging (LI), and chest imaging (CI) according to years since
initial treatment (q = every __ months). BSG = British Sarcoma Group, ESMO = European Society for Medical Oncology,
NCCN = National Comprehensive Cancer Network

justified in patients who are eligible associated risks and benefits to

for curative treatments when PMs
are detected early.
Further studies investigating
these topics can be anticipated;
however, even with increasing data
to inform protocols, determining
appropriate surveillance strategies
will always rely to some extent on
individualized decision making.
Therefore, it remains important for
physicians to understand the
apply published evidence and
guidelines accordingly.
Acknowledgment
The authors would like to thank
Benjamin Miller, MD, MS, for pro-
viding information regarding the
Sarcoma Surveillance AUC and Or-
thoguidelines App.
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